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Background: The Dandy-Walker Malformation (DWM) is a congenital birth malformation that is characterized by a triad of features: cerebellar dysgenesis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa that displaces the dural sinuses and the tentorium. Despite this defining triad, clinical presentation can be highly heterogeneous in part due to severity of structural changes. To date, there been limited consideration of cognitive-behavioral symptoms of DWM in relation to nonmotor functions of the cerebellum, specifically cerebellar cognitive affective syndrome (CCAS). Method: In this case study, we describe the neuropsychological and behavioral profile of a 48-year-old man with DWM who was seen due to concerns, expressed solely by the patient's father, about his son's atypical housing, employment and social skills. Results: Neuropsychological test findings revealed high average intellect on standard intellectual measures (WAIS-IV), with stronger verbal (superior) than perceptual reasoning (average) skills. Across all cognitive domains, performance was generally within expectations, although bilateral fine motor skills were impaired. In contrast, he exhibited weaknesses on nontraditional neuropsychological measures assessing orbitofrontal-limbic circuitry, including reward sensitivity decision making and indices of threat-related emotional physiology. Conclusions: Through the use of traditional and nontraditional neuropsychological measures, subtle cognitive weaknesses in fronto-executive and affective regulation were illuminated and likely explain the patient's functional difficulties. Etiologically, these findings are consistent with the nonmotor functions of the cerebellum as described by CCAS.

Objective : Many patients require repeat neuropsychological evaluations to determine change over time. Repeat evaluations lead to practice effects, which can impact the validity of the assessment. The current study assessed, in older adults, the validity of an alternative set of verbal memory stories created by Newcomer and colleagues. Method : A total 154 of non-demented adults, ages 60-92, completed the WMS-III logical memory (LM) stories and two Newcomer stories (Carson-Jones) as part of a larger battery of neurocognitive tests. The Carson-Jones stories were scored for: (1) verbatim (traditional) and (2) thematic (developed for this study) accuracy. Story memory variables were compared to each other and additional neurocognitive measures using bivariate correlations. A subset of participants ( n =  133) completed magnetic resonance imaging (MRI) and various structural regions (e.g. thickness and volume of medial temporal lobe structures) were used to assess external validity of Carson-Jones stories with hierarchical multiple regression analyses. Results : There was a strong positive correlation between WMS-III LM and Carson-Jones stories for both verbatim and thematic scoring. Both scoring types showed convergent validity with other verbal memory measures (e.g. WMS-III LM and HVLT-R Delay/Learning) and divergent validity with Stroop Word Reading and JOLO. Regarding neuroimaging correlates, Carson-Jones verbatim scoring was significantly associated with left subiculum and left whole hippocampal volume whereas thematic scoring was significantly associated only with left subiculum. Conclusions : Newcomer stories appear to be a valid alternative to WMS-III LM stories in terms of assessing verbal memory in healthy older adults.

Background: Essential tremor is a highly prevalent movement disorder characterized by kinetic tremor and mild cognitive-executive changes. These features are commonly attributed to abnormal cerebellar changes, resulting in disruption of cerebellar-thalamo-cortical networks. Less attention has been paid to alterations in basic emotion processing in essential tremor, despite known cerebellar-limbic interconnectivity., Objectives: In the current study, we tested the hypothesis that a psychophysiologic index of emotional reactivity, the emotion modulated startle reflex, would be muted in individuals with essential tremor relative to controls., Methods: Participants included 19 essential tremor patients and 18 controls, who viewed standard sets of unpleasant, pleasant, and neutral pictures for six seconds each. During picture viewing, white noise bursts were binaurally presented to elicit startle eyeblinks measured over the orbicularis oculi., Results: Consistent with past literature, controls' startle eyeblink responses were modulated according to picture valence (unpleasant > neutral > pleasant). In essential tremor participants, startle eyeblinks were not modulated by emotion. This modulation failure was not due to medication effects, nor was it due to abnormal appraisal of emotional picture content., Conclusions: Neuroanatomically, it remains unclear whether diminished startle modulation in essential tremor is secondary to aberrant cerebellar input to the amygdala, which is involved in priming the startle response in emotional contexts, or due to more direct disruption between the cerebellum and brainstem startle circuitry. If the former is correct, these findings may be the first to reveal dysregulation of emotional networks in essential tremor., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Exercise "stress tests" are widely used to assess cardiovascular function and to detect abnormalities. In line with the view of exercise as a stressor, the present study examined the relationship between cognitive function and cardiovascular activity before and after light physical exercise in a sample of 84 non-demented community-dwelling older adults. Based on known relationships between hypertension, executive function and cerebral white matter changes, we hypothesized that greater post-exercise reactivity, as indexed by higher pulse pressure, would be more related to worse performance on frontal-executive tasks than pre-exercise physiologic measures. All participants were administered a comprehensive neuropsychological battery and underwent a Six Minute Walk Test (6MWT), with blood pressure (BP) measures obtained immediately before and after the walk. Pulse pressure (PP) was derived from BP as an indicator of vascular auto-regulation and composite scores were computed for each cognitive domain assessed. As predicted, worse executive function scores exhibited a stronger relationship with post-exercise PP than pre-exercise PP. Results suggest that PP following system stress in the form of walking may be more reflective of the state of vascular integrity and associated executive dysfunction in older adults than baseline physiologic measures.

Studies with healthy elderly adults suggest that apathy, depression, and anxiety are more common among individuals with mild cognitive impairment (MCI). This study examined differences in mood/amotivational symptoms among patients with Parkinson's disease (PD) with and without MCI. Patients with PD (N=214) underwent neurocognitive evaluations including assessment of apathy (Apathy Scale), depression (Beck Depression Inventory-II), and trait anxiety (State-Trait Anxiety Inventory trait scale). Trait anxiety and depression were more severe in patients with PD with MCI. Delineation of MCI into amnestic and nonamnestic subtypes revealed greater depression, apathy, and anxiety among patients with PD with amnestic MCI relative to cognitively intact patients with PD. Patients with PD and MCI report greater mood symptoms compared to patients with PD who are cognitively intact.

Due to controversy regarding the influence of apathy on quality of life (QoL), the authors examined the independent influence of apathy, depression, and trait anxiety in a nondemented sample of patients with Parkinson disease (PD). Participants (N=107) completed standard self-report measures of QoL and mood/motivation. Analyses investigated the contribution of these measures and empirically derived factor scores on QoL. QoL was predicted by trait anxiety, dysphoria, and decreased interest, with no independent contribution of apathy. Different patterns emerged with respect to domain-specific QoL, with trait anxiety being the strongest predictor across most domains. Anxiety was most widely related to QoL in PD, with minimal contribution of apathy. Future studies should examine different roles of PD mood/motivation symptoms on caregiver QoL.

Perceptual learning is gaining acceptance as a potential treatment for amblyopia in adults and children beyond the critical period. Many perceptual learning paradigms result in very specific improvement that does not generalize beyond the training stimulus, closely related stimuli, or visual field location. To be of use in amblyopia, a less specific effect is needed. To address this problem, we designed a more general training paradigm intended to effect improvement in visual sensitivity across tasks and domains. We used a "global" visual stimulus, random dot motion direction discrimination with 6 training conditions, and tested for posttraining improvement on a motion detection task and 3 spatial domain tasks (contrast sensitivity, Vernier acuity, Glass pattern detection). Four amblyopic macaques practiced the motion discrimination with their amblyopic eye for at least 20,000 trials. All showed improvement, defined as a change of at least a factor of 2, on the trained task. In addition, all animals showed improvements in sensitivity on at least some of the transfer test conditions, mainly the motion detection task; transfer to the spatial domain was inconsistent but best at fine spatial scales. However, the improvement on the transfer tasks was largely not retained at long-term follow-up. Our generalized training approach is promising for amblyopia treatment, but sustaining improved performance may require additional intervention.

Introduction: The Parkinson's disease questionnaire-39 (PDQ-39) is a common measure of health related quality of life (HRQoL) that is widely used with Parkinson disease (PD) patients. Previous evidence suggests that the PDQ-39 reflects at least 8 dimensions (i.e., Emotion, Cognitions, Mobility, etc). To date, little research has examined the external/convergent validity of the Cognitions and Emotional Well-being domains of the PDQ-39., Methods: A convenience sample of 303 PD patients underwent a comprehensive multi-domain neuropsychological evaluation, including tests of execution function, episodic verbal memory, processing speed, language and working memory, as well as completing measures of depression, apathy, state and trait anxiety and HRQoL (PDQ-39). Hierarchical regressions were conducted in order to examine the relationship between scores on neuropsychological tests and the Cognitions index, as well as mood measures and the Emotional Well-being index of the PDQ-39., Results: Neuropsychological test performance did not account for a significant amount of variance in the PDQ-39 Cognitions index scores. Instead, it was depression that significantly contributed to the Cognitions index, above and beyond neuropsychological performance. The PDQ-39 Emotional Well-being index was also related to mood measures, primarily depression and trait anxiety., Conclusions: The PDQ-39 Cognition index may be more related to mood functioning, as opposed to cognitive functioning, and should not be considered a "proxy" for cognitive functioning. Future studies are needed to better explain the construct of this index., (Copyright © 2014 Elsevier Ltd. All rights reserved.)